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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Myeloproliferative syndrome and the myeloproliferative diseases were used in the past to describe myeloproliferative neoplasms. For cases diagnosed 2010 and forward, although the term "myeloproliferative syndrome" is not currently used to describe this disease, the synonyms "myeloproliferative syndrome" and "myeloproliferative disease" were added to the database for myelodysplastic/myeloproliferative neoplasm, unclassified [9975/3].

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code grade to 6 [B-cell] for the histology malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant [9680/3]. Under the Definition section for histology code 9680/3 it states there are morphologic variants of the disease: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.

Rule G3 in the Heme Manual confirms the grade listed in the Heme DB under its Grade section for the histology 9680/3. While the patient presented with a variant of DLBCL that is T-cell/histiocyte rich, it is still a B-cell phenotype. The grade is coded accordingly.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per Rule M11, this case is to be accessioned as two primaries; follicular lymphoma, grade 1 [9695/3] and diffuse large B-cell lymphoma (DLBCL) [9680/3]. The case represents a chronic neoplasm (follicular lymphoma, grade) and an acute neoplasm (diffuse large B-cell lymphoma) diagnosed within 21 days of one another and there is documentation of two biopsies, one confirming the chronic disease and the other confirming the acute disease.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code the histology to 9591/3 [B-cell lymphoma, NOS] per Rule PH28 which states that one is to code the histology when the diagnosis is

• specific histology

• specific histologies
• Heme DB Multiple Primaries Calculator documents the specific histologies and NOS are the

There is only one non-specific histology code mentioned, low grade B-cell lymphoma. This term is synonymous with B-cell lymphoma, NOS.

Per the Multiple Primaries Calculator, when comparing the histology 9591/3 [B-cell lymphoma, NOS] and 9671/3 [lymphoplasmacytic lymphoma], it is the same primary. When comparing the histology 9591/3 [B-cell lymphoma, NOS] and 9699/3 [marginal zone lymphoma], it is the same primary.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Thrombocytopenia NOS is not a reportable diagnosis per Appendix F. Thrombocytopenia and Refractory Thrombocytopenia are not the same disease.

Thrombocytopenia is caused by a decreased number of platelets in the blood. Non-malignant causes include disseminated intravascular coagulation (DIC), drug-induced non-immune thrombocytopenia, drug-induced immune thrombocytopenia, hypersplenism, immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura, and infections of the bone marrow.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code the primary site to C421 [bone marrow]. Our hematopoietic specialty physicians state that involvement of peripheral blood is equivalent to bone marrow involvement because the marrow produces blood. In the absence of any other involvement, per Module 7 (Coding primary sites for lymphomas) Rule PH26, it states to code the primary site to bone marrow when the only involvement is bone marrow.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per Rule PH28, code histology to 9650/3 [Classical Hodgkin lymphoma]. This rule states to code the non-specific (NOS) histology when the diagnosis is one non-specific (NOS) histology and two or more specific histologies.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. http://seer.cancer.gov/seertools/hemelymph.